International Journal For Multidisciplinary Research

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Prion Disease in Animals and Humans: Exploring, Pathogenesis, Transmission

Author(s) Ms. Amanpreet Kaur, Ms. Tanvi Sawhney, Mr. sanjiv duggal
Country India
Abstract Prion diseases are fatal neurodegenerative disorders affecting both humans and animals, caused by misfolded prion proteins that induce the conversion of normal cellular prion protein into pathogenic forms. This misfolded protein resist degradation, accumulates in the brain, and induces neuronal death, leading to rapid cognitive and motor decline. This project aims to investigate the mechanisms of pathogenesis, transmission pathways, and early detection methods for these transmissible spongiform encephalopathy (TSEs). Stanley Prusiner’s team advanced this “protein-only hypothesis “, coining the term prion (1982) as a nucleic acid –resistant proteinaceous particle by 1985. A total of 27,872 cases of prion disease (PrD) and 24,623 cases of Creutzfeldt-Jakob disease (sCJD) have been reported across 34 countries with accessible annual data. Prion disease, including Creutzfeldt-Jakob disease (CJD), fatal insomnia and Kuru, can occur sporadically, be inherited, or result from exposure to infected material. Although rare, these disease are invariably fatal, with no current cure available. Recent advancement in diagnostic methods, such as Real-Time Quaking-Induces conversion (RT-QuIC) and Magnetic Resonance Imaging (MRI), offers hope for earlier diagnosis. Emerging treatment approaches, including antisense oligonucleotides (ASOs) and tetracyclic compound’s, provide optimism for possible therapies. By synthesizing current knowledge and identifying gaps in research, this project seeks to contribute to a deeper understanding of prion diseases and inform future diagnostic and therapeutic developments.
Keywords prion disease, neurodegenerative disorders, protein folding, Prpc, PrPSc, Creutzfeldt-Jakob disease, antisense oligonucleotides, tetracyclic compounds.
Field Medical / Pharmacy
Published In Volume 7, Issue 2, March-April 2025
Published On 2025-04-30
DOI https://doi.org/10.36948/ijfmr.2025.v07i02.42809
Short DOI https://doi.org/g9g73m
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